Respiratory problems have a significant impact on morbidity and mortality in patients with cerebral palsy (CP). In particular, recurrent aspiration, impaired airway clearance, spinal and thoracic deformity, impaired lung function, poor nutritional status, and recurrent respiratory infections negatively affect respiratory status. Bronchopulmonary dysplasia may contribute to pulmonary problems, but asthma is not more common in CP than in the general population. We discuss treatment options for each of these factors. Multiple coexisting and interacting factors that influence the respiratory status of patients with CP should be recognized and effectively addressed to reduce respiratory morbidity and mortality.
What this paper adds
Respiratory problems are a significant cause of morbidity in patients with cerebral palsy (CP).
Respiratory status in patients with CP is influenced by recurrent aspiration and impaired airway clearance.
Spinal and thoracic deformity, impaired lung function, poor nutrition, and respiratory infections also negatively affect respiratory status.
These factors should all be addressed to reduce respiratory problems in patients with CP.
Studies that provide solid evidence of the effectiveness of different airway clearance techniques in patients with COPD are rather scarce. The available evidence indicates that active breathing techniques, such as active cycle of breathing techniques, autogenic drainage and forced expiration, can be effective in the treatment of COPD. The evidence for passive techniques such as postural drainage and percussion is low. Supporting techniques such as intrapulmonary percussive ventilation, positive expiratory pressure and non-invasive ventilation have little evidence because of the small number of studies. Little evidence is found for the combined use of active techniques and supporting techniques such as (oscillating) positive expiratory pressure, postural drainage and vibration in COPD patients. There is clearly a need for well-powered controlled clinical trials on the long-term effects of (combined) airway clearance techniques in COPD.
Background: Functional respiratory imaging (FRI) is a quantitative postprocessing imaging technique used to assess changes in the respiratory system. Using FRI, we characterized the effects of the long-acting muscarinic antagonist (LAMA), glycopyrrolate metered dose inhaler (GP MDI), and the long-acting β2-agonist (LABA), formoterol fumarate metered dose inhaler (FF MDI), on airway volume and resistance in patients with moderate-to-severe chronic obstructive pulmonary disease. Methods: Patients in this phase IIIb, randomized, double-blind crossover study received twice-daily GP MDI (18 μg) and FF MDI (9.6 μg). Primary endpoints were specific (i.e. corrected for lobar volume) image-based airway volume (siVaw) and specific image-based airway resistance (siRaw), measured using FRI. Secondary and other endpoints included additional FRI, spirometry, and body plethysmography parameters. Postdose efficacy assessments were performed within 60–150 min of dosing on day 15. Results: A total of 23 patients were randomized and 19 completed both treatment periods. GP MDI and FF MDI both achieved significant improvements from baseline to day 15 in siVaw [11% ( p = 0.0187) and 23% ( p < 0.0001) increases, respectively] and siRaw [25% ( p = 0.0219) and 44% ( p < 0.0001) reductions, respectively]. Although, on average, improvements were larger for FF MDI than GP MDI, some individuals displayed greater responses with each of the two treatments. These within-patient differences increased with airway generation number. Spirometry and body plethysmography endpoints showed significant improvements from baseline in inspiratory capacity for both treatments, and numeric improvements for other endpoints. Conclusion: Both GP MDI and FF MDI significantly improved siRaw and siVaw at day 15 versus baseline. FRI endpoints demonstrated increased sensitivity relative to spirometry and body plethysmography in detecting differences between treatments in a small number of patients. Intra-patient differences in treatment response between the LAMA and the LABA provide further support for the benefit of dual bronchodilator therapies. ClinicalTrials.gov registration number: NCT02937584 The reviews of this paper are available via the supplemental material section.
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